The utility of high-frequency ultrasonography in preoperative assessment of vulvar Paget’s disease – a case report

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The utility of high-frequency ultrasonography in preoperative assessment of vulvar Paget’s disease – a case report

Case report

J Ultrason 2019; 79: 311–313. doi: 10.15557/JoU.2019.0047

© Polish Ultrasound Society. Published by Medical Communications Sp. z o.o. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial- NoDerivatives License (CC BY-NC-ND). Reproduction is permitted for personal, educational, non-commercial use, provided that the original article is in whole, unmodified, and properly cited.

The utility of high-frequency ultrasonography in preoperative assessment of vulvar Paget’s disease – a case report

Michał Migda

, Marian Stanisław Migda

, Bartosz Migda

, Robert Krzysztof Mlosek

, Marek Maleńczyk

1 Clinical Unit of Obstetrics, Women’s Disease and Gynecological Oncology, United District Hospital, Collegium Medicum University of Nicolaus Copernicus in Toruń, Torun, Poland

2 Civis Vita Medical Center Torun, Poland

3 Department of Diagnostic Imaging, Second Faculty of Medicine with the English Division and the Physiotherapy Division, Medical University of Warsaw, Warsaw, Poland

Correspondence: Michał Migda, Warszawska 20, 87-100 Toruń; Poland, e-mail: mchmigda@gmail.com

DOI: 10.15557/JoU.2019.0047 Abstract

Paget’s disease of the vulva is a rare skin cancer accounting for less than 1% of all vulvar neo- plasias. Surgery is the first treatment of choice. Unfortunately, it is insufficient in many cases, which require further treatment, and the risk of recurrence is high. We present a case of an 85-year-old woman who underwent surgery due to Paget’s disease of the vulva. A preopera- tive skin imaging using a high-frequency 48 MHz mechanical probe was used to assess the lesion margins. Typical high-frequency ultrasonographic features of vulvar Paget’s disease were identified and analyzed. This is the first report of high-frequency ultrasonography imaging of vulvar Paget’s disease.

Submitted:

15.11.2019 Accepted:

22.11.2019 Published:

31.12.2019

Keywords Paget’s disease,

vulva, high-frequency ultrasonography, ultrasound

Case report

An eighty-five-year-old patient was referred to our unit with a diagnosis of PDV after biopsy. The main symptoms were discomfort and itching for over 3 months. The patient had arterial hypertension and glaucoma. A few years ago she suffered from encephalitis complicated with right facial paralysis. Physical examination revealed acetow- hite areas in the left labia minora and majora with spread to left thigh (Fig. 1). The patient was qualified for partial left sided vulvectomy. Before the surgical procedure, we used DermaView high-frequency mechanical probe scan- ner (Dramiński S.A., Poland) to evaluate the PDV margins.

The characteristic ultrasonographic features of malignant lesion were hypoechoic lesion and altered normal HFUS image of the vulva⁽⁷⁾ as compared to adjacent tissue, that is, the dermis with hypoechoic subepidermal band (Fig. 2, Fig. 3, Fig. 4). The lesion was within the dermis with blurred irregular outer margins. Additionally, we noticed

Introduction

Paget’s disease of the vulva (PDV) is a rare superficial skin cancer accounting for less than 1% of all vulvar neopla- sias⁽¹⁾. Extramammary manifestation occurs in the apo- crine glands and is a form of intraepidermal adenocar- cinoma^(2,3). The most commonly affected regions are the vulva, perineum, perianal region, scrotum, penis, or pubic area⁽⁴⁾. Although surgery is the most effective treatment, a complete excision is challenging due to PDV intraepithe- lial reticular spread⁽²⁾. According to recent data, the rate of involved margins after primary surgery is up to 72%⁽⁵⁾. High-frequency ultrasonography (HFUS) is a technological advance that has been used for over 30 years in dermatol-

ogy^(6-8). Although the value of HFUS as a method for differ-

ential diagnosis of skin cancer is still debatable, it gives a clear picture of the size and depth of the tumor, playing an important role in preoperative evaluation⁽⁹⁾. Therefore, we would like to present the utility of HFUS in PDV.

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edematous and enlarged blood vessels (Fig. 2, Fig. 4). We also measured skin thickness at the PDV site, margin and healthy skin, which were 4.38 mm, 3.48 mm and 2.17 mm, respectively. The margin was usually irregular, but we found no deeper invasion into the subcutaneous tissue.

Compression with the ultrasound probe did not change the shape of the tissue. Although we could assess the margin of PDV, it was not possible to excise the entire lesion for technical reasons. Immunohistochemistry was positive for cytokeratin 7 in neoplasmatic cells (Fig. 5). The margin was involved, confirming incomplete excision.

Discussion

From all cases reported in the literature, this is the first one with a preoperative assessment of PDV using HFUS. Since it is a rare cutaneous intraepithelial adenocarcinoma that originates from the apocrine glands, the depth of invasion is the most important prognostic factor. Tumors with invasion of >1 mm spreading outside the skin or into lymph nodes or other tissues are associated with poorer prognosis⁽¹⁰⁾. The optimal management of PDV remains unclear. Surgical exci- sion is usually the primary therapy⁽¹¹⁾.

As in our case, surgical excision often results in positive margins due to anatomical limitations. In addition, the disease is often multifocal and many patients require multiple excisions resulting in significant morbidity⁽¹²⁾. Currently, researchers have gained the possibility to exam- ine skin with novel high resolution imaging technique known as HFUS. The possibility of real-time imaging and measurement, mobility and the lack of contraindications make it a helpful tool in skin diagnosis. In dermatological oncology, HFUS is used to assess the depth of invasion in melanomas and basal cell carcinomas (BCC)⁽¹³⁾. Similarly to PDV, preoperative determination of surgical margins determines future management and patient’s prognosis⁽¹²⁾. Polańska et al. reported that melanomas and BCC have a similar presentation in the form of hypoechoic circular or oval structures surrounded by hyperechoic areas⁽¹³⁾. In the case of PDV, the high-frequency image is also hypoechoic with irregular shape. Additional features included a sub- epidermal low-echogenicity band and blurred outer edges with increased blood vessel diameter and edema. In our opinion, these features could be used for better preopera- tive assessment and planning. In our case, HFUS imaging indicated that the margin would be PDV positive, which was later confirmed by histology. Hopefully, in cases of PDV with more favorable location or smaller size, the HFUS could improve surgical outcome in terms of nega- tive margins. Unfortunately, in most PDV cases reported in the literature, significant delay in the diagnosis leads to quite extensive vulvar lesions and more unfavorable outcome⁽¹⁴⁾.

Conclusions

HFUS may serve as a useful tool in the diagnosis of Paget’s disease of the vulva. We were first to present Fig. 1. Paget’s disease of the vulva on the left labia majora and thigh

erythematous or eczematous, focally eroded or crusted pla- ques (arrows)

Fig. 2. Hypoechoic lesion with edema and enlarged blood vessels (arrow). The margin is blurred. The disease extends to the subcutaneous tissue (long arrow)

Fig. 3. Paget’s disease of the vulva – blurred margin (long arrows) and reaches the subcutaneous tissue

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The utility of high-frequency ultrasonography in preoperative assessment of vulvar Paget’s disease – a case report

Conflict of interest

Authors do not report any financial or personal connections with other persons or organizations, which might negatively affect the contents of this publication and/or claim authorship rights to this publication.

the characteristic ultrasonographic features of PDV.

It seems that the routine use of HFUS in preoperative assessment and planning is possible due to its mobil- ity and general acceptance with no contraindications.

Further research in this area is recommended to con- firm our report.

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Fig. 4. Subepidermal low-echogenicity band (long arrow) with en-

larged blood vessels (thick arrows) Fig. 5. Immunohistochemistry with positive cytokeratine 7 in neo- plasmatic cells (arrow)